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A total of 66 children and adolescents with chronic kidney disease (CKD) (20 pre-dialysis patients and 46 chronic dialysis patients) were evaluated to address the prevalence of abnormalities in glucose and insulin metabolism and their association with cardiovascular disease. Glucose intolerance was assessed using an oral glucose tolerance test; insulin resistance was estimated by the homeostasis model assessment of insulin resistance (HOMA-IR). Carotid artery intima-media thickness (IMT) and left ventricular hypertrophy (LVH) were examined as early markers of cardiovascular disease. Thirty-four patients (7 pre-dialysis, 27 dialysis) exhibited an abnormal glucose tolerance; however, ten patients (7 pre-dialysis, 3 dialysis) were insulin-resistant. Height-specific standard deviation scores of carotid artery IMT were above normal in three of the pre-dialysis patients (15%) and in 34 of the dialysis patients (74%). LVH was present in seven pre-dialysis (35%) and 34 dialysis patients (74%). In addition, two of the pre-dialysis patients (10%) and 18 of the dialysis patients (39%) had severe LVH. The prevalence of both increased carotid artery IMT and LVH were similar in patients with or without glucose intolerance in both groups, but severe LVH was more prevalent in pre-dialysis patients with glucose intolerance (p = 0.042). The multivariate analyses showed that neither carotid artery IMT nor LVM index was predicted by serum glucose levels or HOMA-IR. In conclusion, children with CKD are at a high risk of glucose intolerance and also have a greater risk of subclinical cardiovascular disease (CVD). However, the presence of glucose intolerance does not appear to be an independent risk factor for increased carotid artery IMT or LVH.

To determine the incidence of latent tuberculosis infection and evaluate the follow-up protocol of the patients diagnosed with juvenile idiopathic arthritis (JIA) and other chronic rheumatologic diseases treated with anti-TNF-α treatment (etanercept, infliximab, adalimumab) in Turkey, 144 patients were evaluated retrospectively for the development of tuberculosis. Patients were evaluated every 6 months for tuberculosis using history, physical examination, tuberculin skin test (TST), chest radiographs, and, when required, examination of sputum/early morning gastric aspirates for acid-fast bacilli and chest tomography. A tuberculin skin test over 10 mm induration was interpreted as positive. Patients were diagnosed with JIA (n = 132), enthesitis-related arthritis (ERA; n = 14), juvenile psoriatic arthritis (JPsA; n = 4), chronic idiopathic uveitis (n = 4), and chronic arthritis related to FMF (n = 8). Mean age was 12.25 ± 3.96 years (4.08-19.41 years), mean duration of illness was 5.86 ± 3.77 years (0.66-15 years), and the mean duration of anti-TNF-α treatment was 2.41 ± 1.47 years (0.6-7 years). Anti-TNF-α agents prescribed were etanercept (n = 133), infliximab (n = 30), and adalimumab (n = 6). When unresponsive to one anti-TNF-α therapy, patients were switched to another. There was no history of contact with individuals having tuberculosis. During follow-up, seven patients (4.8%) with positive TST were given INH prophylaxis. One oligoarticular JIA patient (0.69%) diagnosed with secondary uveitis who had been followed for 5 years and had been using infliximab for 2 years, developed a positive Quantiferon-TB test while on INH prophylaxis. He was started on an anti-tuberculosis drug regimen. In conclusion, anti-TNF-α treatment in children with chronic inflammatory disease is safe. Follow-up every 6 months of children on anti-TNF-α treatment with respect to tuberculosis by the pediatric infectious disease department is important to prevent possible complications.

The aim of the study was to determine the economical impact of juvenile idiopathic arthritis (JIA) and familial Mediterranean fever (FMF) in Turkey. A total of 100 patients (69 F/31 M) with JIA and 100 with FMF (68 F/32 F) who were consecutively seen in the outpatient clinic of the pediatric rheumatology department at Cerrahpasa Medical School between August 2008 and January 2009 were studied. Cost data were collected through a questionnaire filled out by the parents. The mean age (JIA: 11 +/- A 5 years; FMF:12 +/- A 4 years) and mean disease duration (JIA:5 +/- A 3 years; FMF: 4 +/- A 3 years) of the patients were similar. JIA patients were assigned to 5 subtypes (polyarticular: n = 45, oligoarticular: n = 30, systemic onset: n = 13, psoriatic: n = 6, and enthesopathy-related JIA: n = 6). Forty-nine percent of the patients with JIA were treated with anti-TNF drugs and 61% with DMARDs. All patients with FMF were using colchicine. The total annual cost of JIA (a,not sign3,994 +/- A 4,101) was considerably higher than that of FMF (a,not sign162 +/- A 77) (P < 0.001). Medication fee was the major determinant of total costs in both diseases constituting 85% in JIA and 39% in FMF. Among the subtypes of JIA, total annual costs were the highest among patients with polyarticular type (a,not sign6,045 +/- A 4,078). Medications especially anti-TNF drugs were the major contributor among all determinants of costs in JIA. The low costs of health care system and prominent changes in the health care policies for the last 5 years in Turkey might have played role in our findings.

The clinical course and symptoms of connective tissue diseases in nbsp; adolescence is different nbsp; from the other childhood periods Both diseases and clinical courses of diseases show significant variations in this period nbsp; In this article it nbsp; is intended to review connective tissue diseases that can be seen in the adolescence Turk Arch Ped nbsp; 2011; 46 Suppl: 118 27

The aim of this study was to evaluate longitudinal changes in dialysate cancer antigen 125 (dCA125) levels over time and to analyze relationships between dCA125 and peritoneal glucose exposure (PGE) in children undergoing long-term peritoneal dialysis (PD). The study group included seven boys and four girls (mean age 13 ± 5.1 years) with a mean PD duration of 84.0 ± 1.1 months. A peritoneal equilibration test (PET) was performed, and dCA125 levels were measured in all patients. Peritoneal appearance rates (AR) of dCA125, the velocity of the decrease in dCA125AR values, and annual PGE levels were also calculated. The final tests were performed at a mean of 63.3 ± 3.5 months after the initial ones. Both dCA125 and dCA125AR levels showed statistically significant decrements during the follow-up period (p = 0.003), with the velocity of decrease in dCA125AR found to be 52.6 ± 19.4%. There were no significant differences in peritoneal transport parameters between the beginning and end of the study period. PGE values were significantly higher in the last year of the study than in the first year (p = 0.014), but the velocity of the decrease in dCA125AR levels was not related to total PGE. In conclusion, a significant decline was found in dCA125 and CA125 AR levels, reflecting mesothelial cell mass, in children undergoing long-term PD (>5 years), but these were not related to PGE.

We report a rare case of polyarteritis nodosa (PAN) presenting in childhood. The child had multiple visceral aneurysms and later developed ascending colitis and jejunitis. The diagnosis was established with multidetector CT and CT angiography.

Coronary artery calcification (CAC) is common in adults with chronic kidney disease (CKD) and progresses with time. However, data are limited for younger patients. We have previously reported CAC in eight of 53 children with CKD. After 2 years, CAC evaluation was repeated in 48 patients. The median CAC score (CACS) increased from 101.3 (1473.6 +/- 1978.6, range 8.5-4332) to 1759.2 (2236.4 +/- 2463.3, range 0-5858) Agatston units (AU). When the individual changes in CACS were evaluated one by one, we showed a mild decrease in two patients on hemodialysis (HD) and in one transplant (Tx) recipient, a moderate increase in one patient on HD, one on peritoneal dialysis (PD) and one Tx recipient, and a large increase in one HD patient. Also, CAC disappeared in one HD patient. All patients with no calcification at baseline remained calcification-free at follow-up. To obtain the individual cumulative exposure, we calculated time-averaged mean values, using the laboratory values from the beginning of dialysis to the first and second multidetector spiral computed tomography (MDCT) scans (baseline and final values, respectively). Final CACS was positively related to final calcium-phosphorus (CaxP) product, while CAC progression was inversely associated with final serum albumin level. This report is the first study with the largest number and the youngest cohort to document the natural history of coronary calcification.

Cardiovascular complications are common in patients with end-stage renal disease (ESRD). We aimed to investigate left ventricular (LV) function and carotid intima-media thickness (cIMT) in children and adolescents with ESRD. This study included 38 ESRD patients (15 hemodialysis and 23 peritoneal dialysis) and 17 age- and sex-matched healthy subjects. The ESRD patients had significantly lower mean mitral E/A ratio, and higher left ventricular mass index (LVMI) and cIMT than the control group. Compared with PD patients, HD patients had worse LV diastolic function. In stepwise linear regression analysis, LVMI (P = 0.043) and hemoglobin (P = 0.015) turned out to be independent variables for predicting diastolic dysfunction (reduced E/A ratio), and the only significant predictor of cIMT was indexed diastolic blood pressure (DBP) (P = 0.035). Cardiovascular structure and function abnormalities are also common in pediatric dialysis patients, as in adults. Furthermore our data indicated that hemodialysis was disadvantageous for preserving LV diastolic function as compared with peritoneal dialysis.

Antiphospholipid syndrome is an autoimmune disease characterized by recurrent thrombosis and the presence of antiphospholipid antibodies. Clinical presentations are dependent on the affected vessels and organs. The most common presentation of antiphospholipid syndrome is arterial or venous thrombosis. An unusual presentation of the disease is characterized by microvascular thrombosis with multiorgan involvement, which is termed catastrophic antiphospholipid syndrome. The diagnosis of catastrophic antiphospholipid syndrome can be difficult because of the heterogeneity of the different clinical forms. Clinical manifestations of catastrophic antiphospholipid syndrome are complex with multiple organ involvement, resulting in renal insufficiency, heart failure, acute respiratory distress syndrome, and liver involvement. Early diagnosis and aggressive therapies are essential in this condition because of the extremely high mortality rate. Herein, the case of a 14-year-old girl with catastrophic antiphospholipid syndrome that was previously misdiagnosed as a vasculitis related to parvovirus B19 infection is presented.

The aim of our study was to show how the progression and severity of Familial Mediterranean Fever (FMF) is affected by procoagulant activity and alterations in the markers of thrombosis and fibrinolysis. The study cohort comprised 64 FMF patients who were classified as attack-free patients (Group 1; n = 34 patients, aged 3-19 years) and attack patients (Group 2; n = 30 patients, aged 3-21 years). All patients were on colchicine treatment with the exception the newly diagnosed patients in Group 2. A total of 14 healthy subjects between 5-12 years of age were enrolled as controls (Group 3). Laboratory tests, including leukocyte and thrombocyte counts, erythrocyte sedimentation rate, CRP, fibrinogen, PT, aPTT, Factor VIII, vW factor, D-dimer, P-selectin, tPA and PAI-1, were carried out on all patients. Inflammation continued both during the attack and attack-free period in FMF. The prolongation of PT was observed during attacks (PT = 13.6 s in Group 2, and PT = 12.6 s in Group 3; p = 0.002). tPA levels increased in FMF patients (tPA levels of group 1, 2 and 3 were 12.6, 13.2 and 9.7 ng/ml, respectively; p = 0.01). P-selectin was lower in both patient groups than in the control group. During attack periods PAI-1 levels increased (PAI-1 level of Group 1: 89.6 ng/ml and PAI-1 level of Group 2: 335.7 ng/ml, p = 0.000). Inflammation with increased acute phase reactants continued during both attack and attack-free periods in FMF patients. Prolongation of PT and differences in tPA and P-selectin levels suggest that hypercoagulability may have a role in the etiopathogenesis of FMF. It may be possible to use PAI-1 as a marker for the attacks of FMF.

Coronary artery calcification (CAC) is common in adults with end-stage renal disease (ESRD), but little is known about the prevalence and the extent of it in children. We used multidetector spiral computed tomography (MDCT), echocardiography, and carotid and brachial high-resolution ultrasonography to screen for the presence and predisposing factors of CAC in 53 children with ESRD [15 hemodialysis (HD) patients, 24 peritoneal dialysis (PD) patients, and 14 renal transplant (rTx) recipients]. CAC was present in 15% of patients (three HD patients, three PD patients, and two rTx). The mean age of the patients with CAC was 16.4 years (range: 11.0–21.2 years), and their median CAC score was 101.3, ranging from 8.5 to 4,322 according to the Agatston method. The patients with CAC had longer duration of total dialysis (P=0.005), had higher time-integrated serum phosphorus (P

This prospective study was conducted to assess dialysate cancer antigen 125 (dCA125) levels in pediatric peritoneal dialysis (PD) patients and to investigate whether it exhibits any alterations during or after recovery from peritonitis, and also to analyze the relationships between dCA125 and age, duration of PD treatment, peritonitis incidence, time passed since the last episode of peritonitis, PD prescription parameters, and peritoneal transport parameters. Forty-seven standardized 4-h peritoneal equilibration tests (PET) were performed in 38 children (mean age 11.6+/-4.7 years) on PD (duration 20.9+/-14.3 months). Thirty-two of the patients were in stable condition at the time of PET (stable group). Six patients were included in the study only during a peritonitis episode, and two of the stable patients were reevaluated during a peritonitis episode afterwards (peritonitis group). Seven out of a total of eight patients with acute peritonitis were reexamined after recovery (recovery group). CA125 levels were measured in 188 samples at 0-, 1-, 2-, and 4-h dwells. Peritoneal appearance rates (AR) were calculated. Mean dCA125 (4 h) and AR CA125 values were 5.6+/-5.3 U/ml [median 4.15 U/ml/4 h (range 0.5-25.9)] and 50.1+/-45.6 U/min/1.73 m2[median 37.91 U/ml/1.73 m2 (range 3.61-223.39)]. AR CA125 levels did not correlate with age, PD duration, peritonitis incidence, time passed since the last peritonitis episode, exchange volume used per m2 per day, or peritoneal transport properties in the stable patients' group. Although stable patients using hypertonic PD solutions (n=16) had slightly lower AR CA125 levels (p=0.04), multivariate analysis showed no influence of hypertonic dextrose solutions on mesothelial CA125 secretion (p=0.4). During acute peritonitis, CA125 concentrations showed a reversible threefold increase [AR CA125: stable 37.9 vs. peritonitis 101.2 U/ml/1.73 m2 (p=0.001)]. No difference could be found between the stable group and the recovery group. We conclude that changes in the peritoneal mesothelial cell mass cannot be assessed by determining CA125 in a cross-sectional way and that longitudinal determinations could be more valuable in the follow-up of patients.

Objective To characterize pediatric patients who had been diagnosed with polyarteritis nodosa (PAN) through necrotizing vasculitis of the small and mid-size arteries or those with characteristic findings on angiograms data were collected. Study design Pediatricians were asked to classify their patients into one of the four suggested groups for juvenile PAN. Twenty-one pediatric centers worldwide participated with 110 patients. Results The girl:boy ratio was 56:54, with a mean age of 9.05 ± 3.57 years. The cases were classified as: 33 (30%) cutaneous PAN; 5 (4.6%) classic PAN associated with hepatitis B surface antigen (HBs Ag); 9 (8.1%) microscopic polyarteritis of adults associated with antineutrophil cytoplasmic antibodies (ANCA); and 63 (57.2%) systemic PAN. Cutaneous PAN was disease confined to the skin and musculoskeletal system. All patients with HBs Ag–associated classic PAN were diagnosed with renal angiograms. Antiviral treatment was administered in most cases. Microscopic PAN patients had pulmonary-renal disease, in combination or separately. ANCA was present in 87%, and 2 patients progressed to end-stage renal failure. Patients classified with systemic PAN had multiple system involvement, almost all had constitutional symptoms, and all had elevated acute phase reactants. Corticosteroids and cyclophosphamide were the first choices of immunosuppressive treatment. The overall mortality was 1.1%. Conclusions There were remarkable differences among pediatric patients with PAN, with different clinical manifestations and overall better survival and lower relapse rates when compared with adults.

The aim of this study was to investigate the adequacy of the following criteria for the classification of juvenile-onset spondyloarthropathies (JSpA): European Spondyloarthropathy Study Group criteria (ESSGCr) and Amor criteria (ACr) (proposed for adult SpA), Garmisch-Partenkirchen (G-PCr), seronegative enthesopathy and arthropathy syndrome (SEACr), and atypical spondyloarthropathies classification criteria (ASpCr) (proposed for JSpA). Sixty-two patients with JSpA (48 male and 14 female) and 64 with juvenile idiopathic arthritis (27 male and 37 female) (excluding enthesitis-related and psoriatic arthritis) were enrolled in the study group. Twenty-nine of the patients with JSpA were diagnosed with definite JSpA and the remaining 35 with undifferentiated JSpA. One hundred six patients in the study group were evaluated by one investigator, who was unaware of the diagnosis, according to the following: ESSGCr, ACr, G-PCr, ASpCr, and SEACr. Analysis of the patients diagnosed with JSpA showed 83.9%, 82.3%, 95.2%, 61.3%, and 62.9% sensitivity and 87.5%, 95.3%, 78.1%, 98.4%, and 92.2% specificity for the ESSGCr, ACr, G-PCr, ASpCr, and SEACr sets, respectively. None of the criteria evaluated above is sufficient for the classification of JSpA. There is a definite need for a new set of criteria with high specificity and sensitivity for early recognition and classification.

A defect in MEFV gene expression regulation has been implicated in familial Mediterranean fever (FMF) etiopathophysiology. Here we show significantly higher expression level in second exon lacking MEFV transcript in FMF patients compared with healthy controls (P=0.026). Our results also point out a possible role of exon 2 deleted MEFV transcript in FMF pathogenesis.

ObjectiveTo investigate the prevalence of MEFV gene mutations in Turkish patients with Henoch-Schönlein purpura (HSP) but with no symptoms of familial Mediterranean fever (FMF). In addition, we assessed the clinical and laboratory characteristics of HSP patients with and without MEFV mutations.MethodsEighty pediatric patients with HSP (44 boys and 36 girls) were enrolled. Blood for mutation analysis was obtained either at the time of the diagnosis of HSP or during followup visits in previously diagnosed patients. No patient had the diagnosis of FMF in their history and in the followup period. Exon 10 of the MEFV gene was screened, together with p.E148Q mutation analysis.ResultsTwenty-seven (34%) patients were found to be heterozygous for one of the screened MEFV mutations; p.M694V in 16, p.M680I in 5, p.V726A in 3, and p.E148Q in 3 patients. Patients with MEFV mutations were younger than those without mutations and they had edema and arthritis more frequently. Also, the frequencies of elevated erythrocyte sedimentation rate and C-reactive protein values were found to be significantly higher in patients who had MEFV mutations.ConclusionAlterations in the MEFV gene are important susceptibility factors for the development of HSP and also affect the clinical presentation of it.

Mediyastinal lipomatoz (ML), mediyastende aşırı yağ birikimine bağlı ortaya çıkan ve ender görülen bir durumdur (1). Sıklıkla endojenik ya da iyatrojenik Cushing Sendromu ya da şişmanlık ile ilişkilidir (2,3). Mediyastinal lipomatoz olguları, hiçbir klinik bulgu vermeyeceği gibi, aşırı yağ birikimine bağlı ortaya çıkan ve ciddi bası bulgularına neden olan klinik bulgularla da karşımıza çıkabilir. Ön-arka göğüs grafisinde görülen mediyastende iki taraflı genişleme en sık radyolojik bulgudur ve mediyastene ait kitleler ile karıştırılabilir (1). Kesin tanı, bilgisayarlı tomografi (BT) veya manyetik rezonans (MR) görüntüleme ile konur (4,5). Steroide bağlı ML genellikle iyi seyirlidir ve steroid dozunun azaltılması ya da kesilmesi ile geriler. Buna karşılık ciddi bası bulgularına yol açan ve cerrahi girişim gerektiren olgular literatürde bildirilmiştir (5,6). Biz de bu olgu nedeni ile sistemik steroid kullanımının ender görülen bu komplikasyonuna dikkat çekmek istedik., To the Editor Mediastinal lipomatosis ML is a rare condition caused by excessive fat accumulation in the mediastinum 1 It is frequently related to endogeneous or iatrogenic Cushing syndrome or obesity 2 3 Patients with mediastinal lipomatosis may be asymptomatic or may present with clinical findings which are caused by excessive fat accumulation and may lead to severe compression findings The most common radiologic finding is bilateral enlargement in the mediastinum on postero anterior chest graphy and can be confused with mediastinal masses 1 The definite diagnosis is made by computarized tomography CT or magnetic resonance imaging MR 4 5 Steroid related ML generally has a good prognosis and improves when the dose of steroid is decreased or steroid treatment is discontinued However patients with severe compression findings who need surgical intervention have been reported in the literature 5 6 We wanted to draw attantion to a rare complication of systemic steroid use with this case A 17 year old female patient who was diagnosed as systemic lupus erytemathosus SLE and whose renal biopsy was compatible with stage IV diffuse membranoproliferative glomerulonephritis had received multiple intravenous high dose methylprednisolone intravenous cyclophosphamide and oral prednisolone and azathioprine mycophenalate mofetil MMF treatments because of active disease When she presented for follow up visit she was using 40 mg prednisolone and 1000 mg MMF On physical examination she appeared fallen and pale Moon face bufallo hump and pretibial edema were present Her blood pressure was found to be 150 100 mmHg her pulse rate was found to be 86 min and her respiratory rate was found to be 20 min She had no peripheral lympadenopathy or organomegaly A 2 6 systolic murmur was heard at the mesocardiac area Other system examinations were found to be normal Laboratory tests revealed a deep anemia hemoglobin: 6 5 g dL haptoglobin: lt;28 5 mg dL hyoalbuminemia serum albumin: 2 6 g dL proteinuria urinary protein: 2 1 g day and renal dysfunction serum creatinine: 2 2 mg dL On antero posterior chest graphy bilateral mediastinal enlargement was observed Figure 1 Echocardiography revealed pericardial effusion 6 mm and hypertension secondary to left ventricular hypertrophy findings Thoracic CT without contrast was performed with a suspicion of mediastinal mass and widespread fat accumulation was found Figure 2 No mediastinal adenomegaly or mass was observed Sterioid related ML was considered in the patient who had a history of long term use of high dose steroid and the dose of steroid was reduced up to 10 mg but steroid treatment could not be discontinued since the disease had an active course On the follow up graphy taken 4 weeks later enlargment in the mediastinum was observed to be regressed Mediastinal lipomatosis is a rare complication of systemic steroids and was described by Koerner and Sun 1 in 1966 for the first time As far as we know steroid ndash;related ML has been reported only in one pediatric subject until the present time 7 While some patients have no clinical findings some others may have severe compression findings related to airways vena cava superior heart and other organs 6 8 9 10 Our patient had no clinical finding suggesting compression of ML However ehcocardiography of our patient revealed pericaridal effusion When the cases in the literature were evaluated it was found that Hsu et al 11 reported a case of pericardial effusion related to ML However we think pericardial effusion can not be explained with ML alone in our patient who was being followed up with a diagnosis of SLE and who had hypoalbuminemia The diagnosis of mediastinal lipomatosis is generally made by observation of mediastinal enlargement on chest graphy and demonstration of intensity changes related to fat on CT or MR imaging In our patient mediastinal enlargement was observed initially and CT was performed considering secondary malignancy because of a history of long term and intensive immunosupressive treatment A diagnosis of ML was made by demonstrating widespread fat mass on CT ML was thought to be related to steroid in our patient who was known to have used long term steroid and who had clinical findings related to steroid use moon face and bufallo hump Regression of mediastinal enlargement with reduction of steroid dose supported the diagnosis Conclusively ML is a rare complication which should be considered in the differential dignosis in patients who hava a history of systemic steroid usage and mediastinal enlargement Nur Canpolat1 Sebuh Kuruğoğlu2 Lale Sever1 Salim Çalışkan1 Meltem Kıvılcım3 Özgür Kasapçopur4 Nil Arısoy4 1İstanbul University Cerrahpaşa Medical Faculty Division of Pediatric Nephrology İstanbul Turkey 2İstanbul University Cerrahpaşa Medical Faculty Department of Radiology İstanbul Turkey 3İstanbul University Cerrahpaşa Medical Faculty Department of Pediatrics İstanbul Turkey 4İstanbul University Cerrahpaşa Medical Faculty Division of Pediatric Rheumatology İstanbul Turkey References 1 Koerner HJ Sun DI Mediastinal lipomatosis secondary to steroid therapy Am J Roentgenol Radium Ther Nucl Med 1966; 98: 461 464 2 Nguyen KQ Hoeffel C Le LH Phan HT Mediastinal lipomatosis South Med J 1998; 91: 1169 1172 3 Lee WJ Fattal G Mediastinal lipomatosis in simple obesity Chest 1976; 70: 308 309 4 Enzi G Biondetti PR Fiore D Mazzoleni F Computed tomography of deep fat masses in multiple symmetrical lipomatosis Radiology 1982; 144: 121 124 5 Nguyen KQ Hoeffel C Le LH Phan HT Mediastinal lipomatosis South Med J 1998; 91: 1169 1172 6 Taille C Fartoukh M Houel R Kobeiter H Remy P Lemaire F Spontaneous hemomediastinum complicating steroid induced mediastinal lipomatosis Chest 2001; 120: 311 313 7 Shukla LW Katz JA Wagner ML Mediastinal lipomatosis: a complication of high dose steroid therapy in children Pediatr Radiol 1988; 19: 57 58 8 Sorhage F Stover DE Mortazavi A Unusual etiology of cough in a woman with asthma Chest 1996; 110: 852 854 9 Gombar S Mitra S Thapa D Gombar KK Pathak R Anesthetic considerations in steroid induced mediastinal lipomatosis Anesth Analg 2004; 98: 862 864 10 Bulakci M Yahyayev A Ucar A Erer B Erer B Dursun M Unusual cause of right ventricular outflow tract compression: mediastinal lipomatosis J Thorac Imaging 2011; 26: W134 136 11 Hsu YM Yao NS Liu JM Steroid induced mediastinal lipomatosis with radiographic features of pericardial effusion Am J Emerg Med 2000; 18: 346 348

Amaç: Bu çal›flmada böbrek ifllevleri azalmakta olan çocuklarda glomerül filtrasyon h›z› tayininde serum sistatin C’nin kreatinine göre daha iyi bir de¤iflken olup olmad›¤› araflt›r›ld›. Gereç ve Yöntem: Göztepe E¤itim-Araflt›rma Hastanesi ve Cerrahpafla T›p Fakültesi Çocuk Nefroloji Poliklinikleri’nde izlenen 100 çocuk (53 k›z/47 erkek) çal›flmaya al›nd›. Çal›flmada alt›n standart olarak dietilen triamin penta asetat (DTPA) klirensi al›nd›. Dietilen triamin penta asetat klirensi 80mL/dak/1,73m2’nin üstünde olan olgular birinci gruba (n=64), alt›ndakiler ikinci gruba (n=36) al›nd›. Olgular›n serum sistatin C ve kreatinin düzeyleri ölçüldü, glomerül filtrasyon h›z› Schwartz formülüyle (GFH schw) hesapland›. De¤iflkenlerin tan›sal de¤erleri ROC analizi ile de¤erlendirildi. Tüm çocuklar›n ailelerinden imzalanm›fl ayd›nlat›lm›fl onay formu al›nd›. Bulgular: Olgular›n 53’ü k›z, 47’si erkek olup yafllar› ortalama 8,4±5,1 (0,17-20,5) y›l idi. Birinci gruba 64 olgu, ikinci gruba 36 olgu al›nd›. Olgular›n serum sistatin C ve kreatinin düzeyleri ile GFHSchw aras›nda her iki grupta anlaml› bir iliflki saptand› (p, nbsp

Enzymuria and low molecular weight proteinuria reflect tubular damage and dysfunction, respectively. We examined urinary N-acetyl-β-. D -glucosamini-dase (U-NAG) and β2-mic

Objective: To correlate serum anti-cyclic citrullinated peptide antibodies (anti-CCP) levels with juvenile idiopathic arthritis (JIA) subtypes and with an erosive disease course. Methods: The study group comprised 122 children with JIA; 16 were evaluated during both active disease and remission. Nineteen children with systemic lupus erythematosus (SLE), 27 with rheumatoid arthritis (RA), and 15 healthy children were also included in the study. Twelve children with JIA were rheumatoid factor (RF) positive, and 34 patients had persistent erosive joint disease. Anti-CCP antibody levels were determined by ELISA; values above 5 relative units were regarded as positive. Results: Three girls with seropositive polyarticular JIA and erosive joint disease had positive anti-CCP values. Children evaluated during active disease and remission, patients with SLE, and healthy children all had negative anti-CCP antibody levels. However, 19/27 (70%) adult patients with RA had positive anti-CCP antibody values. Conclusions: In contrast with RA, anti-CCP positivity is only rarely found in patients with JIA. In patients with RF positivity and/or in patients with erosive joint disease, anti-CCP can be detected.

We investigated the efficacy and safety of Hepatitis B vaccine (HBVac) in steroid sensitive nephrotic syndrome (SSNS) children. 41 patients with SSNS and 30 controls were vaccinated with HBVac(Engerix B(®)). Patients were divided into 3 subgroups:full dose steroid users, alternate-day steroid users and steroid non-users. Seroconversion rate was lower in steroid users than non-users at the 6th(p=0.015) and 12th(p=0.036) months. Antibody to Hepatitis B surface antigen(HBsAb) titers were significantly different between subgroups and controls at the 15th month. However, HBsAb and response rates were not different between subgroups at the 12th and 15th months (p>0.05). Five patients were unresponsive to HBVac. Relapse rates after the vaccination were higher than those in the prevaccination period (p=0.002). HBVac is less effective in producing immune response in SSNS children with steroid therapy. HBVac may trigger relapse in some patients. We recommend HBVac to SSNS children with low dose steroid therapy or after steroids are discontinued.

Systemic lupus erythematosus (SLE) is a chronic autoimmune connective tissue disease with multi-organ involvement that is associated with the presence of autoantibodies, and it is influenced by genetic, endocrine, or environmental backgrounds such as exposure to ultraviolet rays and viral infections for the etiopathogenesis [1]. In the cardiovascular system, it frequently causes pericarditis, nonbacterial verrucous endocarditis, coronary artery disease, and cardiomyopathy [2]. Cardiovascular complications in SLE are generally presumed to be associated with inflammatory activity as well as premature development of atherosclerosis [1]. The overall morbidity and death from these complications have been increasing in recent years, and they are now the major cause of death in SLE [3]. Dissecting aortic aneurysm in SLE has been recognized as a rare life-threatening complication, but its pathogenesis is still obscure. The pathogenesis of aortic aneurysms and dissection in SLE has been attributed to aortic circulatory disturbances resulting from vasculitis and cystic medial degeneration as well as to atherosclerosis. The occurrence of atherosclerosis is considered to be due to long-term steroid treatment, hyperlipidemia, hypertension, and nephrotic syndrome [4]. The importance of the high prevalence of premature atherosclerosis and cardiovascular disease in SLE should be addressed, even in young adolescents, and especially those with early childhood-onset disease. Nevertheless, aortic dissection is a life-threatening disorder that requires immediate diagnosis and treatment to prevent mortality. To our knowledge, this is the second adolescent patient with childhood-onset SLE complicated with aortic dissection, but the first case treated with endovascular stent-grafting.

In this study, we examined the antibody responses after recombinant hepatitis B vaccine in juvenile SLE patients and whether antibody levels were affected by immunosuppressive therapy.This study consisted of 64 juvenile SLE patients and 24 healthy controls. We evaluated HBsAg, Anti-HBs and Anti-HbcIgG titers in SLE patients. 24 patients (37%) were non-immunised, 39 patients were immunised (61%) and 1 patient (1.5%) was chronic hepatitis B carrier. Of the 24 non-immunised patients, 3 had active disease (SLEDAI10) and 1 was being treated for tuberculosis infection so they were not included in the vaccination program. Twenty non-immunised SLE patients were given 3 dose recombinant hepatitis B vaccine doses at 0,1,6 months. AntiHBs antibody titer10 IU/ml one month after the last dose of vaccine was accepted as seroconversion.After 3 doses of vaccination, 16 (80%) of SLE patients and all of the healthy controls had seroconversion. Since two patients had SLEDAI score10 after the first 2 doses of vaccine and one patient had SLEDAI score10 after the first dose, these patients were given only two doses of hepatitis B vaccine. These patients had already seroconverted. One patient had exacerbation of the disease one month after the third dose of the vaccine. Protective antibody responses were statistically insignificant between the two groups (p=0.49). Geometric mean antibody titers of SLE patients were lower than those of the healthy controls. Adequate antibody response was not affected by immunosuppressive treatment as prednisone, azathioprine, and hydroxychloroquine.Juvenile SLE patients could reach an adequate antibody response after recombinant hepatitis B vaccination and this response is not affected by immunosuppressive treatment.

SUMMARY: Aim: Cardiovascular abnormalities are common in children with chronic kidney disease (CKD). Left ventricular (LV) structure and functions have been extensively studied by conventional pulse-wave Doppler echocardiography (cPWD), however, tissue Doppler imaging (TDI) is a relatively new echocardiography method. The aims of this study were to evaluate LV diastolic function in paediatric dialysis patients using cPWD and TDI methods, and to compare the findings obtained with two modalities. Methods: This study included 38 children and adolescents on dialysis (14 haemodialysis and 24 peritoneal dialysis, duration of dialysis 58.0 1 32.8 months) and 16 age- and sex-matched healthy subjects. Results: The mean left ventricular mass index (LVMI) was significantly higher in the patient group (P < 0.001) and the most common cardiac geometry was concentric LV hypertrophy (55%). There was no significant difference in LV systolic function between patient and control groups. However, dialysis patients had worse LV diastolic function both according to cPWD (lower E/Aratio) and TDI (lower Em/Am ratio) than the healthy subjects (P < 0.001 and P = 0.001, respectively). Also, the index of LV filling pressure (E/Em ratio) obtained by the combination of cPWD and TDI was significantly higher in the patients (P < 0.001). Cumulative dose of calciumbased phosphate binder (CBPB), diastolic blood pressure and LVMI were the independent predictors of E/Em ratio. Conclusion: Our study shows that LV diastolic dysfunction is common in paediatric dialysis patients and TDI findings correlate well with cPWD findings. Similarly, higher dose intake of CBPB, hypertension and LV hypertrophy have a negative effect on LV filling pressure suggesting diastolic function. nep_1124 636..642

Juvenile idiopathic arthritis (JIA) is a disease that was prominent with increased inflammation response in immune system, appeared mostly with peripheral arthritis and endogenous and exogenous antigens play a role in the pathogenesis of disease. Two major reasons were thinking to be considerably important. First of them is immunological predisposition and the second one is environmental factors. Infections are considered to be the most important between environmental factors but also stress and trauma are also important in the etiology of the disease. However, the relation between JIA and infections is not clearly defined but the relation between adult chronic arthritis and infections was well-defined. A total of 70 patients, 26 with primer JIA, 20 with recurrent JIA, 24 healthy control were included in this study. Mycoplasma pneumoniae, Chlamydophila pneumoniae and C. Jejuni were detected in 4, 1 and 1 of 10 (38.46%) patients with primer JIA, respectively. Salmonella enteritidis, EBV, M. pneumoniae, C. jejuni and Borrelia burgdorferi were detected in 1, 2, 2, 2, and 1 of the 8(40%) patients with recurrent JIA, respectively. S. enteritidis were isolated in feces culture and also identified by agglutination method. Infection was detected in total 18 (39.13%) of patient groups. C. pneumoniae and C. jejuni were detected in 1 and 1 of 2(8.33) healthy control groups, respectively. Throat culture positivity was not detected in any of the patient and healthy control groups. In conclusion, etiopathogenesis of JIA is not clearly understood and suggested that various factors can trigger the disease and it is the most common rheumatoid disease of childhood. However, there are some studies focusing especially on one infectious agent but this is the first study including such a big range of infectious agents in the literature for the microorganisms that can be suggested to have a role in the etiopathogenesis of JIA. We have a conclusion in the light of our results and suggest that some microorganisms can trigger and increase the intensity of clinical situation according to the case. When we evaluate the primer and recurrent JIA groups; M. pneumoniae and C. jejuni come forward and seen common in JIA cases. We also suggest that the pre-diagnosis of microorganisms, which can play a role as primarily or by intervening in the etiopathogenesis of JIA and adding specific antimicrobial therapy to the standard JIA therapy, it is possible to perform new, extended, especially molecular based serial case studies.

39th Annual Meeting of the European-Society-for-Paediatric-Nephrology -- SEP 10-13, 2005 -- Istanbul, TURKEY ozkaya, ozan/0000-0002-0198-1221; Cetinyurek Yavuz, Aysun/0000-0003-4024-8775 WOS: 000266882400012 PubMed: 19439991 Background/Aim: The aim of this retrospective study was to evaluate the presentation, clinical and pathological manifestations and outcome of the Henoch-Schonlein purpura (HSP) nephritis in children. Methods: Clinical and laboratory data of 443 children with HSP nephritis aged between 3 and 16 years from 16 pediatric nephrology reference centers were analyzed retrospectively. The biopsy findings were graded according to the classification developed by the International Study of Kidney Disease in Children (ISKDC). Results: Renal biopsy was performed in 179 of the patients with HSP nephritis. The most common presenting clinical finding in patients who were biopsied was nephrotic range proteinuria (25%) which was followed by nephritic-nephrotic syndrome (23.5%). The biopsy findings according to the ISKDC were as follows: class I: 8.3%; II: 44.1%; III: 36.3%; IV: 6.7%; V: 3.3%; VI: 1.1%. All of the patients who developed end-stage renal disease had nephritic-nephrotic syndrome at presentation. Of 443 patients, 87.2% had a favorable outcome and 12.8% had an unfavorable outcome. The overall percentage of children who developed end-stage renal disease at follow-up was 1.1%. Logistic regression analysis did not show any association of initial symptoms and histology with outcome. Conclusion: In the presented cohort, the presence of crescents in the first biopsy or presenting clinical findings did not seem to predict the outcome of HSP nephritis in children. We conclude that children with HSP nephritis even with isolated microscopic hematuria and/or mild proteinuria should be followed closely. Copyright (C) 2009 S. Karger AG, Basel European Soc Paediat Nephrol

Background Juvenile idiopathic arthritis (JIA) is a complex, multifactorial and chronic inflammatory disease of unknown etiology with considerable variability in which tumor necrosis factor-alpha (TNF-α) plays an important role. Both genetic and environmental factors can contribute to a susceptibility to disease initiation as well as a severity of disease course. TNF-α polymorphisms may be an independent marker of susceptibility and severity of JIA. The aim of this study was to elucidate putative association between the -863 C/A polymorphism in the promoter region of the TNF-a gene and susceptibility to onset and severity of JIA.

Results There was no statistically significant difference in between the disease subtype groups and between the pain groups as to the sociodemographic features. The frequency of depression and anxiety diagnosis which were made according to the DSM-IV criteria was %31 and %17.7 in 45 JIA patients. For the 45 patients the average CDI score was 7.60 and the average Trait Anxiety Inventory score was 34.31. For the 42 patients which were aloocated into pain groups, the average CDI score was 7.67 and the average Trait Anxiety Inventory score was 34.10. There was no statistically significant difference between the groups as to the depression and anxiety scale scores. Pain was not correlated with depression and anxiety scores.

The aim of this study was to adjust dry weight by short-term blood volume monitoring (BVM)-guided ultrafiltration and evaluate the effects of optimizing dry weight on blood pressure (BP) control and intradialytic symptoms (IDS) in a group of hypertensive hemodialysis (HD) patients. The study was performed in four sequential phases, each of which lasted for 1 week, on nine hypertensive HD patients (six girls, age 16.9 +/- 3.1 years). In phase I, patients were observed by BVM. In phase II, BVM was used to guide ultrafiltration to adjust dry weight. Antihypertensive drugs were gradually tapered or withheld in phase III, when the patients were hypotensive and/or their IDS increased. In phase IV, this particular weight was maintained without any intervention. Pre- and post-HD body weight, pre-HD, post-HD, 30 min after HD casual BP values, and IDS in each HD session were recorded. The BP was also assessed by 44-h ambulatory BP monitoring (ABPM), which is an ideal method to determine BP changes throughout the interdialytic period at the beginning of phase I and at the end of phase IV. There was a decrease in mean dry weight, all casual systolic BPs, and systolic/diastolic ABPM at the end of the study (all por = 0.05). Antihypertensive drugs were stopped in five patients and reduced in two during phase III of the study. The IDS was more frequent (36%) in phase IV than in phase I (16%); however, this increase did not reach statistical significance. The results of this study suggest that short-term BVM guided-ultrafiltration may be a useful tool to diagnose volume overload and to adjust dry weight and, consequently, to achieve a better control of BP in pediatric HD patients.

Takayasu's arteritis is a chronic inflammatory disease that primarily involves the aorta and its main branches. Varying degrees of narrowing, occlusion, or dilatation develop in the involved vessel segments. However, dissection of the aorta is quite rare in this disease, and it may develop particularly after angioplasty. We report a very rare case of Takayasu's arteritis with dissection of the abdominal aorta just distal to the origin of the inferior mesenteric artery in a 9-year-old girl. She was treated conservatively with close follow-up. At the end of 1 year's follow-up, the dissection of the aorta did not show progression, and new lesions were not identified. To our knowledge, this patient is the youngest child presented with arterial dissection as the initial manifestation of the disease.

Primary Sjögren syndrome (pSS) is an uncommon disease in childhood. Childhood pSS might have different clinical manifestations than adult pSS. We describe a 13-year-old girl with multiple episodes of bilateral parotid swelling lasting 2 years. Her history included severe arthralgia, local edema, and purpura episodes since 9 years of age. During her 3-week hospitalization, 2 episodes of parotid swelling occurred, which both resolved in 48 hours. Ultrasonography and magnetic resonance images of parotid glands showed parenchymal inhomogeneity related to adipose degeneration and nodular pattern. Investigations showed elevated erythrocyte sedimentation rate, the presence of hypergammaglobulinemia, positive antinuclear antibody, and elevated rheumatoid factor, anti—Sjögren syndrome antigen A, and anti—Sjögren syndrome antigen B. Histopathologic examination of labial minor salivary glands revealed focal periductal lymphocytic infiltrate and sialoduct ectasia. She was diagnosed as having pSS. Recurrent parotid swelling is a more characteristic feature of disease in children, and this finding should alert the clinician to the possible diagnosis of pSS.

Objectives To test the hypothesis that alterations in the Mediterranean fever (MEFV) gene are a susceptibility factor for the development of polyarteritis nodosa (PAN) we investigated the prevalence of MEFV mutations in patients with PAN without any symptoms of familial Mediterranean fever (FMF). Study design Pediatric patients with PAN (n = 29) were enrolled in this study. Six predominant mutations (p.M694V, p.M680I, p.M694I, p.V726A, p.K695R, p.E148Q) in the MEFV gene were studied. Results Fifteen MEFV mutations were identified in 58 chromosomes. Eleven of the 29 patients (38%) were found to carry MEFV mutations. Three (10.3%) of them had homozygous p.M694V mutation, and one of the patients (3.4%) had compound heterozygous mutation (p.V726A/p.E148Q). Conclusions Our study confirms that alterations in the MEFV gene are important succeptibility factors for the development of PAN. We believe that mutations in MEFV gene provide a basis for the development of PAN both by forming a proinflammatory state and by possibly giving exaggerated response to streptococcal infections.

Cardiovascular disease (CVD) is the principal cause of mortality in patients with end-stage renal disease (ESRD). The aim of this study was to analyze carotid intima-media thickness (cIMT), endothelium-dependent dilatation (EDD), and left ventricular mass index (LVMI) as the cardiovascular risk markers and to investigate the independent risk factors of these markers in pediatric dialysis patients. This study included 39 children and adolescents undergoing dialysis (15 hemodialysis and 24 peritoneal dialysis) and 15 age- and gender-matched healthy subjects. The cIMT and EDD were assessed by high-resolution ultrasound, and LVMI was calculated from standard echocardiographic measurements. Compared with control subjects, cIMT standard deviation scores (SDS), LVMI, total homocysteine (tHcy), and high-sensitivity C-reactive protein (hs-CRP) values were significantly higher in patients, but EDD values did not differ. The mean hs-CRP level was significantly higher in hemodialysis (HD) patients than in peritoneal dialysis (PD) patients. The cIMT-SDS and LVMI were associated with several variables in univariate analysis. Stepwise linear regression analysis, indexed SBP (p = 0.017), and hemoglobin (p = 0.001) turned out to be independent variables for predicting LVMI, and a significant predictor of cIMT was indexed diastolic blood pressure (DBP) (p = 0.035). The causes of atherosclerosis and left ventricular hypertrophy are multifactorial in children and adolescents with ESRD. Better management of hypertension and anemia may be priorities for preventing or improving CVD in these patients.

The aim of this study was to evaluate the incidence of anemia detected in familial Mediterranean fever (FMF) and the effect of disease activity and colchicine therapy along with interleukins to laboratory tests, including serum transferrin receptor (TfR), in the diagnosis of anemia seen in FMF in children. After detecting anemia in 63.4% of 172 FMF patients followed up by our rheumatology outpatient polyclinics, it was decided to study 3 groups of patients: group 1, 17 newly diagnosed FMF patients; group 2, 36 FMF patients on colchicine therapy; and group 3, 17 healthy children as control for the symptom of anemia. All 3 groups of patients were investigated for their hematological parameters, iron status, including soluble transferrin receptor (sTFR) concentrations and sTFR index, and IL-6 levels. Anemia ratio was 9/17, 53%; 11/36, 31%; and 1/17, 5% in the groups 1, 2 and 3, respectively. There was a significant difference between hemoglobin (Hb) values in the first group and the second (patients who were on colchicine therapy). Furthermore, in the second group there was a significant difference between the Hb concentrations at the time of diagnosis and after colchicine therapy (p = .003). There was a positive correlation between Hb and plasma iron and transferrin saturation in group 1 and disease beginning age, iron, transferrin saturation, and erythrocyte sedimentation rate (ESR) in the second group. In the first group the anemic patients' iron and transferrin saturation were significantly lower than normal, while ferritin levels were higher. In the second group, a good correlation was found with ESR and Hb levels; the higher ESR values were detected in patients with lower Hb values. Of the anemic and nonanemic patients of the first and second groups, values for interleukin 6 and iron parameters, including sTFR, were found similar. Anemia detected in FMF patients was found related to iron status more than interleukins. Colchicine therapy had a positive effect on anemia as well as on disease activity. Resolution of symptoms of FMF occurred with correction of the anemia, if the patient ESR values also decreased on colchicine therapy.

Systemic lupus erythematosus (SLE) is a rheumatologic disease characterized by an inflammatory destruction of the target organ systems of the body in an unknown way by autoantibodies formed against self-antigens. Infectious agents like Epstein–Barr virus (EBV), cytomegalovirus and parvovirus B19 may have a role in the occurrence or the exacerbation of the SLE. In this report, the clinical follow-up of a 14-year-old girl diagnosed with SLE following an EBV infection with bicytopenia, lymphadenomegaly and hepatomegaly is discussed. This case could support the role of viral infections in the etiology of SLE.

Secondary hyperparathyroidism and anemia are the hallmarks in uremic patients. It is suggested that parathyroid hormone increases erythrocyte osmotic fragility and induces hemolysis. The present study was undertaken to examine the possible relationship between erythrocyte osmotic fragility and secondary hyperparathyroidism in 20 pediatric patients on maintenance peritoneal dialysis. We found that erythrocyte osmotic fragility in these patients was normal. No correlation between erythrocyte osmotic fragility and hematochemical changes associated with secondary hyperparathyroidism was found. We conclude that erythrocyte osmotic fragility was normal in pediatric patients on peritoneal dialysis and excess parathyroid hormone levels do not affect erythrocyte osmotic fragility and do not cause anemia.

This study was conducted to determine the frequency of antinuclear antibodies (ANA) positivity and uveitis in our newly diagnosed juvenile idiopathic arthritis (JIA) patients classified according to International League Against Rheumatology (ILAR) classification criteria. Ninety-two girls and 106 boys, totally 198 children were enrolled in the study. of them 36 (18.2 percent) were found to be ANA positive. Chronic anterior uveitis was detected in 20 (10.1 percent) patients. ANA positivity was determined in 4 of the systemic JIA patients, in whom no uveitis had been detected. Twenty-five of 37 patients with oligoarticular JIA were ANA positive, in 10 of them uveitis was also diagnosed. ANA were positive in 3 of 34 patients with RF positive polyarticulat JIA, only one patient had positive ANA, and another one had uveitis. Nine patients were extended JIA and in none of them, ANA positivity or uveitis were present. Of 43 patients classified as enthesitis related arthritis (ERA), uveitis was diagnosed in 6 and there was no evidence of ANA positivity, but one had uveitis. We conclude that the incidence of ANA positivity and uveitis is low in Turkish children with JIA.

To assess aerobic fitness and exercise capacity in patients with juvenile idiopathic arthritis (JIA) and to determine subgroup differences.Thirty-four patients diagnosed with JIA and 21 healthy sedentary volunteers were studied. Aerobic fitness was determined by measuring peak power and peak oxygen uptake (VO2peak) during an incremental cycling test. The patient group consisted of systemic JIA (n = 8), polyarticular JIA (n = 13), oligoarticular JIA (n = 7), and enthesitis-related arthritis (ERA, n = 6). Results from different subgroups of JIA were compared to determine subgroup differences.All subjects tolerated maximal exercise testing well. The JIA group had lower aerobic fitness than controls. In our comparison of JIA subgroups, we found no significant differences in cardiopulmonary measures. The ERA group had higher aerobic capacity than other subgroups. There was no difference in exercise capacity between patients with active disease (n = 10) and those in remission (n = 24).We suggest that heterogeneity in VO2peak levels among JIA patients is due to subgroup differences. Exercise programs for improvement of aerobic fitness should be individualized or at least be modified according to different subgroups.

The role of Chlamydophila pneumoniae in the development and exacerbation of juvenile idiopathic arthritis (JIA) was investigated. Blood samples were taken from 60 JIA patients during an active disease period and for 4 weeks after. Synovial fluid samples were obtained from 20 of the 60 patients. In addition, 22 patients with familial Mediterranean fever (FMF) during the active period and 35 healthy children were included in the study as control groups. Synovial fluid samples were also obtained from three children with FMF. IgG, IgM and IgA levels against C. pneumoniae in serum samples were studied by immunofluorescence and IgG antibody and PCR studies were performed for C. pneumoniae DNA in synovial fluid samples. Twenty-nine (48.3 %) patients with JIA, 18 (81.8 %) patients with FMF and 22 (62.8 %) healthy children were found to be pre-infected with C. pneumoniae. Pre-infection with C. pneumoniae among FMF patients was found to be significantly higher than among those with JIA. We did not find a significant difference between JIA patients and healthy children. Chronic C. pneumoniae infection was observed only in six JIA patients, one FMF patient and two healthy children. Synovial fluid antibodies were found at higher than 1/512-fold dilution in one JIA patient and four times higher than normal serum in three JIA patients. C. pneumoniae DNA was not detected in any synovial fluid sample from FMF or JIA patients by PCR. In conclusion, C. pneumoniae infection does not have a triggering or a progressive effect on the clinical situation in JIA aetiopathogenesis, as a result of a multifactorial aetiology. New, extensive and serial studies (especially PCR studies of synovial tissue) are needed in order to confirm the indirect results.

This study investigates whether renal damage occurs in children with juvenile idiopathic arthritis (JIA) either secondary to the disease per se or due to the side effects of non-steroidal anti-inflammatory drugs (NSAIDs) and slow-acting anti-rheumatic drugs (SAARDs) used in treatment. In this cross-sectional study, albuminuria, N -acetyl glucosaminidase (NAG), beta(2)-microglobulin (beta(2)M), and creatinine (Cr) levels were measured in urine samples of 45 patients (23 female, 22 male, 9.4+/-3.9 years) with JIA and a sex- and age-matched control group of 33 healthy children. The urinary albumin/Cr, NAG/Cr, and beta(2)M/Cr ratios of children with JIA and of the control group did not differ statistically. No difference was noted between patient groups with different types of JIA (12 systemic, 18 polyarticular, and 15 oligoarticular JIA). JIA patients with active disease (n=16) had higher NAG/Cr values than patients with inactive disease (P=0.002). NAG/Cr levels correlated with erythrocyte sedimentation rate (r=0.66, P

In this study bone status was assessed using a quantitative ultrasound (QUS) technique at the calcaneus in 141 healthy, prepubertal, Turkish schoolchildren (80 girls, 61 boys) aged 6-12 years. Broadband ultrasound attenuation (BUA, DB/MHz) was measured with a prototype pediatric contact bone analyzer (CUBA, McCue Ultrasonics Ltd). The relation of age, body weight and height to BUA was assessed. BUA increased linearly with age in boys and girls (R=0.448, p=0.0001 and R=0.382, p=0.002, respectively). BUA values in boys tended to be higher than in girls, reaching significance only at the age of seven years with a 95% confidence interval. In conclusion, the measurement of BUA in the calcaneus with QUS has important clinical implications in assessing bone mass in children. Further studies in not only healthy children but also in those with metabolic bone diseases would be helpful in order to evaluate its sensitivity and reproducibility.

Many therapeutic agents and toxins can produce liver lesions, such as hepatitis, cirrhosis and portal hypertension. It has been reported that 10% of cases of apparent hepatitis are due to drug-induced injury. 1 Drugs produce cytotoxic, cholestatic or mixed forms of acute hepatic injury with features of both hepatocellular and cholestatic jaundice. Up to 25% of cases of fulminant hepatic failure might be the result of adverse reaction to medications. 1

There have been conflicting reports concerning the effect of carnitine supplementation on lipid metabolism in patients on peritoneal dialysis (PD). We investigated several parameters of lipid metabolism in pediatric PD patients supplemented with carnitine. The study included 20 patients receiving PD (treatment group) aged 2-18 years and a matched healthy control group. In the treatment group, baseline triglyceride, total cholesterol, low-density lipoprotein cholesterol, very low-density lipoprotein cholesterol, and apolipoprotein B levels were higher than in the control group. High-density lipoprotein cholesterol, free fatty acid, phospholipids, and apolipoprotein A-I levels were not different from those in the control group. The baseline plasma free carnitine level was lower and acyl-carnitine level was higher in the treatment group. No difference was found between the groups with respect to plasma total carnitine levels. Oral l-carnitine supplementation (50 mg/kg per day for 30 days) led to a significant decrease (from a baseline value of 146.6+/-51.8 mg/dl to 63.6+/-22.2 mg/dl, P0.001) in apolipoprotein B levels, and no significant change in the other lipid parameters of the treatment group. Oral l-carnitine supplementation does not ameliorate the lipid profile in pediatric PD patients, but it causes a significant decrease in apolipoprotein B levels. Hence, carnitine supplementation may be recommended for decreasing apolipoprotein B levels in this patient population.

Juvenile dermatomyositis (JDM) is an uncommon vasculitis in childhood. JDM is the most prevalent idiopathic inflammatory myopathies of childhood. JDM is characterized by proximal muscle weakness and typical skin involvement.

Background Crohn’s disease and ulcerative colitis are multifactorial and heterogeneous, chronically relapsing inflammatory bowel diseases (IBD) that are thought to result from a dysregulated mucosal immune response to gut lumen bacterial antigens in a genetically susceptible host (1). Additional clinical and epidemiological evidence supported MEFV as a potential IBD candidate gene. Familial Mediterranean fever (FMF) and IBD share common clinical and biologic features; they are both inflammatory disorders characterized by the same chronic relapsing behavior, infiltration by neutrophils at the site of injury, and abnormal regulation of apoptosis (2,3). Objectives Incidense of these two disease in Turkish children, relation with the existing MEFV gene mutation, prognosis of the disease and response to the treatement and accompanying laboratory findings were evaluated. Methods Patients who have two or more of these symptoms which are fever, abdominal pain, chest pain, arthritis and family history of FMF are diagnosed as FMF according to criterias defined in 2009 (4).We evaluated complaints, physical examinations, MEFV gene mutations,erythrocyte sedimantation rate,C reactive protein,complete blood counts of the FMF patients during the first admission to our clinic.In order to evaluate IBD colonoscopy was applied to the patients if they had any of these findings which are bloody stool, chronic diarrhea, abdominal pain inappropriate for FMF, positive ASCA IgA, IgG and pANCA.Diagnosis of IBD was made according to the colonoscopy findings and histopathological study of the biopsy samples. Results 46% of patients was male, FMF diagnostic age was 7.5±3.4 year,application age was 9.75±4.8 year.M694V, M680I, E148Q, R202Q, K695R mutations of MEFV gene were studied in all patients.One of these mutations was positive in 84% of the patients.Colonozcopy applied to 15 patients who have complaints of abdominal pain inappropriate to FMF,chronic diarrhea, bloody diarrhea and/or positive pANCA, ASCA and biopsy samples were taken.6 patients had moderate and 3 patients had mild colitis findings on histopathologic samples.All of the 9 patients have MEFV gene mutation.M694V homozygote mutation was found in 3 of these 9 patients.M694V heterozygote mutation in 1 patient,K695R homozygote mutation in 1,K695R heterozygote mutation in 1,M680I heterozygote in 1 and E148Q heterozygote mutation in 1 patient were detected. Conclusions The incidence of IBD during childhood increases if patients have FMF disease. This result suggests that either of these diseases accompany frequently or they are parts of the whole which is consisting of the same genetic mutations. References Podolsky DK (2002) Inflammatory bowel disease. N Engl J Med 347: 417–29. Lichtenberger GS, Flavell RA, Alexopoulou L. Innate immunity and apoptosis in IBD. Inflamm Bowel Dis 2004; 10: 58–62. McDermott MF (2004) A common pathway in periodic fever syndromes. Trends Immunol 2004; 25: 457–60. Yalcinkaya F, Ozen S, Ozcakar ZB et al. A new set of criteria for the diagnosis of familial Mediterranean fever in childhood. Rheumatology 2009; 48: 395-98. Disclosure of Interest None Declared

Objective: To evaluate the responsiveness of children with juvenile idiopathic arthritis ( JIA) to pneumococcal vaccine and to determine whether the response was influenced by disease subtype and immunosuppressive treatment. Methods: 50 patients with JIA (all in remission) and 24 healthy children were included in the study. All children were vaccinated with 23-valent pneumococcal vaccine. Serum samples were collected from each child at the beginning and six weeks after the vaccination in order to determine anti-pneumococcal capsular polysaccharide (PCP) IgG levels by ELISA. A two-fold or more rises in antibody titer after the vaccination was considered as positive immune response. Results: Six weeks after the vaccination 46 (92%) children with JIA and 23 (95.8%) healthy children developed an effective antibody response. This vaccine responsiveness was not different between two groups (p=0.34). The anti-PCP IgG levels of healthy children were significantly higher than anti-PCP IgG levels of children with JIA at the beginning and after the vaccination (p=0.004 and p=0.029). Conclusion: Children with JIA had an adequate response to pneumococcal vaccine and the response was influenced by MTX and prednisone treatment. The vaccine should be undertaken during the time period when the child receiving the smallest possible dose of the immunosuppressive therapy.

Background and aim Systemic lupus erythematosus (SLE) have accelerated atherosclerosis and increased risk of cardiovascular disease. It is thought that the atherosclerosis process begins in the first decades of life and progresses silently. The aim of this study is to evaluate subclinical cardiovascular abnormalities including atherosclerosis and left ventricular hypertrophy (LVH) in children and adolescent with SLE.